Apremilast in benign chronic pemphigus (Hailey–Hailey disease)

Loughlin, Constanza Riquelme‐Mc; Iranzo, Pilar; Mascaró, José M.

doi:10.1111/ced.14225

Cited by 12 publications

(13 citation statements)

References 8 publications

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“…4,5 Few reports have been published on patients with refractory HHD who experienced marked improvement after treatment with apremilast, 2,3 however, it is not always successful. This lack of response has been noted in other series such as Riquelme-Mc Loughlin et al 6 and question the magnitude of the positive results seen to date (Table 1). Clinical response to apremilast may be limited to those locations where sweating is a well-known aggravating factor, hence the importance of complementing the treatment with other rescue therapies.…”

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confidence: 47%

Apremilast in combination with botulinum toxin‐A injection for recalcitrant Hailey‐Hailey disease

et al. 2021

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confidence: 47%

Apremilast in combination with botulinum toxin‐A injection for recalcitrant Hailey‐Hailey disease

et al. 2021

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“…Furthermore, Yoto et al reported two cases of HHD treated with apremilast with no recurrence for up to 2 years (Yoto et al, 2021). On the other hand, Riquelme-Mc Loughlin et al (2020) used apremilast with five patients, but none of them showed any improvement. Apremilast was similarly ineffective in Case 2, even though it was less severe than Case 1.…”

Section: Discussionmentioning

confidence: 99%

Case report: Difference in outcomes between two cases of Hailey-Hailey disease treated with apremilast

et al. 2022

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Hailey-Hailey disease (HHD) is a rare autosomal dominant acantholytic dermatosis clinically characterized by recurrent erythematous plaques and erosions mainly on the intertriginous regions. Although HHD seriously affects quality of life, conventional treatments often fail to provide long-term relief for most patients. The effectiveness of apremilast, a phosphodiesterase-4 inhibitor, against severe HHD was first reported in 2018, and after further testing, this agent is currently expected to be established as an efficacious and safe therapeutic option. Here we report two cases of HHD treated with apremilast which showed opposite outcomes. Although the case with extremely severe symptoms showed remarkable and long-lasting improvement with apremilast used after acute treatment with oral corticosteroid, the other case, with milder symptoms treated only with apremilast, showed no improvement. Our transcriptome analysis using skin samples collected prior to apremilast administration revealed the involvement of the NF-κB signaling pathway, which is related to the responses to bacteria and other organisms. However, this pathway was more strongly activated in case 2 than in case 1, suggesting that the steroid treatment preceding apremilast may have been effective and supportive in the apremilast-responding case. One of the two cases highlights the potential of apremilast as a treatment option for HHD, but the other underlines the difficulties in managing HHD and the complexity of the disease background. The accumulation of cases and larger clinical studies are expected to precisely evaluate the safety and efficacy of apremilast, and the potential for therapies in combination with conventional treatments.

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“…To our knowledge, there have been 13 reported cases of HHD treated with apremilast, including our own, as shown in Table 1. [3][4][5][6] None of the previous reports included information regarding any mutation in the ATP2C1 gene. According to the previous reports, the severity of the skin lesion was described in 11 of 13 cases; nine cases were classified as severe and two cases were classified as moderate.…”

Section: Discussionmentioning

confidence: 99%

“…2 Recently, treatment with apremilast for a skin lesion of HHD has been reported. [3][4][5][6] We herein report two cases of HHD effectively treated with apremilast and review the previous literature.…”

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Two cases of Hailey‐Hailey disease effectively treated with apremilast and a review of reported cases

Yoto

Makino

Mizawa

et al. 2021

The Journal of Dermatology

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Hailey-Hailey disease (HHD) is an autosomal dominant genetic disease caused by a mutation of the ATP2C1 gene. 1 It is clinically characterized by the development of recurrent blisters and erosions in friction areas.Topical or systemic corticosteroids, antibiotics, antifungals, systemic cyclosporine, and methotrexate are administered to reduce inflammation and prevent flare-up, but the efficacy is not always sufficient. 1 Apremilast is an oral-administered, small-molecule inhibitor of phosphodiesterase 4 (PDE4) that induces an improvement in psoriasis and psoriatic arthritis. 2 Recently, treatment with apremilast for a skin lesion of HHD has been reported. [3][4][5][6] We herein report two cases of HHD effectively treated with apremilast and review the previous literature.

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Apremilast in benign chronic pemphigus (Hailey–Hailey disease)

Abstract: The current coronavirus disease (COVID-19) has forced the shutdown of many nonessential services in most high-risk countries. Most Dermatology consultations

Cited by 12 publications

References 8 publications

Apremilast in combination with botulinum toxin‐A injection for recalcitrant Hailey‐Hailey disease

Apremilast in combination with botulinum toxin‐A injection for recalcitrant Hailey‐Hailey disease

Case report: Difference in outcomes between two cases of Hailey-Hailey disease treated with apremilast

Two cases of Hailey‐Hailey disease effectively treated with apremilast and a review of reported cases

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